A concept for integrated care pathways for atopic dermatitis-A GA2 LEN ADCARE initiative.

INTRODUCTION: The integrated care pathways for atopic dermatitis (AD-ICPs) aim to bridge the gap between existing AD treatment evidence-based guidelines and expert opinion based on daily practice by offering a structured multidisciplinary plan for patient management of AD. ICPs have the potential to enhance guideline recommendations by combining interventions and aspects from different guidelines, integrating quality assurance, and describing co-ordination of care. Most importantly, patients can enter the ICPs at any level depending on AD severity, resources available in their country, and economic factors such as differences in insurance reimbursement systems. METHODS: The GA2 LEN ADCARE network and partners as well as all stakeholders, abbreviated as the AD-ICPs working group, were involved in the discussion and preparation of the AD ICPs during a series of subgroup workshops and meetings in years 2020 and 2021, after which the document was circulated within all GAL2 EN ADCARE centres. RESULTS: The AD-ICPs outline the diagnostic procedures, possible co-morbidities, different available treatment options including differential approaches for the pediatric population, and the role of the pharmacists and other stakeholders, as well as remaining unmet needs in the management of AD. CONCLUSION: The AD-ICPs provide a multidisciplinary plan for improved diagnosis, treatment, and patient feedback in AD management, as well as addressing critical unmet needs, including improved access to care, training specialists, implementation of educational programs, assessment on the impact of climate change, and fostering a personalised treatment approach. By focusing on these key areas, the initiative aims to pave the way for a brighter future in the management of AD.

Infection with SARS-CoV-2 among children with asthma: evidence from Global Asthma Network.

BACKGROUND: Clinical presentations of coronavirus disease 2019 (COVID-19) among children with asthma have rarely been investigated. This study aimed to assess clinical manifestations and outcome of COVID-19 among children with asthma, and whether the use of asthma medications was associated with outcomes of interest. METHODS: The Global Asthma Network (GAN) conducted a global survey among GAN centers. Data collection was between November 2020 and April 2021. RESULTS: Fourteen GAN centers from 10 countries provided data on 169 children with asthma infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). COVID-19 was asymptomatic in 58 (34.3%), mild in 93 (55.0%), moderate in 14 (8.3%), and severe/critical in 4 (2.4%). Thirty-eight (22.5%) patients had exacerbation of asthma and 21 (12.4%) were hospitalized for a median of 7 days (interquartile range 3-16). Those who had moderate or more severe COVID-19 were significantly more likely to have exacerbation of asthma as compared to those who were asymptomatic or had mild COVID-19 (adjusted odds ratio (adjOR) 3.97, 95% CI 1.23-12.84). Those who used inhaled bronchodilators were significantly more likely to have a change of asthma medications (adjOR 2.39, 95% CI 1.02-5.63) compared to those who did not. Children who used inhaled corticosteroids (ICS) did not differ from those who did not use ICS with regard to being symptomatic, severity of COVID-19, asthma exacerbation, and hospitalization. CONCLUSIONS: Over dependence on inhaled bronchodilator may be inappropriate. Use of ICS may be safe and should be continued in children with asthma during the pandemic of COVID-19.

The Challenges in the Follow-Up and Treatment of Brazilian Children with Hereditary Angioedema.

INTRODUCTION: Hereditary angioedema (HAE) with C1 inhibitor (C1-INH) deficiency is a rare autosomal dominant disease. Although the first symptoms can appear in childhood, the diagnosis's delay has a strong impact on the patient's quality of life. We analyzed clinical and laboratory characteristics and the drug therapy of pediatric patients with HAE in Brazil. METHODS: Medical records from 18 reference centers of HAE patients under 18 years of age were evaluated after confirmed diagnosis was performed by quantitative and/or functional C1-INH. RESULTS: A total of 95 participants (51 M:44 F; mean age: 7 years old) out of 17 centers were included; 15 asymptomatic cases were identified through family history and genetic screening. Angioedema attacks affected the extremities (73.5%), gastrointestinal tract (57%), face (50%), lips (42.5%), eyelids (23.7%), genitals (23.7%), upper airways (10%), and tongue (6.3%). Family history was present in 84% of patients, and the mean delay in the diagnosis was 3.9 years. Long-term prophylaxis (51/80) was performed with tranexamic acid (39/80) and androgens (13/80); and short-term prophylaxis (9/80) was performed with tranexamic acid (6/80) and danazol (3/80). On-demand therapy (35/80) was prescribed: icatibant in 7/35, fresh frozen plasma in 16/35, C1-INH plasma-derived in 11/35, and tranexamic acid in 12/35 patients. CONCLUSIONS: This is the first study on HAE pediatric patients in Latin America. Clinical manifestations were similar to adults. Drugs such as androgens and tranexamic acid were indicated off-label, probably due to restricted access to specific drugs. Educational programs should address pediatricians to reduce late diagnosis and tailored child therapy.

Prevalence, Severity, and Treatment of Recurrent Wheezing During the First Year of Life: A Cross-Sectional Study of 12,405 Latin American Infants

PURPOSE: This study aimed to determine the prevalence and severity of recurrent wheezing (RW) defined as > or =3 episodes of wheezing, risk factors, and treatments prescribed during the first year of life in Latin American infants. METHODS: In this international, cross-sectional, and community-based study, parents of 12,405 infants from 11 centers in 6 South American countries (Argentina, Brazil, Chile, Colombia, Peru, and Uruguay) completed a questionnaire about wheezing and associated risk/protective factors, asthma medications, and the frequency of and indications for the prescription of antibiotics and paracetamol during the first year of life. RESULTS: The prevalence of RW was 16.6% (95% CI 16.0-17.3); of the 12,405 infants, 72.7% (95% CI 70.7-74.6) visited the Emergency Department for wheezing, and 29.7% (27.7-31.7) was admitted. Regarding treatment, 49.1% of RW infants received inhaled corticosteroids, 55.7% oral corticosteroids, 26.3% antileukotrienes, 22.9% antibiotics > or =4 times mainly for common colds, wheezing, and pharyngitis, and 57.5% paracetamol > or =4 times. Tobacco smoking during pregnancy, household income per month <1,000 USD, history of parental asthma, male gender, and nursery school attendance were significant risk factors for higher prevalence and severity of RW, whereas breast-feeding for at least 3 months was a significant protective factor. Pneumonia and admissions for pneumonia were significantly higher in infants with RW as compared to the whole sample (3.5-fold and 3.7-fold, respectively). CONCLUSIONS: RW affects 1.6 out of 10 infants during the first year of life, with a high prevalence of severe episodes, frequent visits to the Emergency Department, and frequent admissions for wheezing. Besides the elevated prescription of asthma medications, there is an excessive use of antibiotics and paracetamol in infants with RW and also in the whole sample, which is mainly related to common colds.

A homozygous CARD9 mutation in a Brazilian patient with deep dermatophytosis.

Deep dermatophytosis has been described in HIV and immunosuppressed patients. Recently, CARD9 (caspase recruitment domain-containing protein 9) deficiency has been reported in individuals with deep dermatophytosis previously classified as "immunocompetent". We report a 24-year-old Brazilian male patient with deep dermatophytosis born to an apparently non-consanguineous family. The symptoms started with oral candidiasis when he was 3 years old, persistent although treated. At 11 years old, well delimited, desquamative and pruriginous skin lesions appeared in the mandibular area; ketoconazole and itraconazole were introduced and maintained for 5 years. At 12 years of age, the lesions, which initially affected the face, started to spread to thoracic and back of the body (15 cm of diameter) and became ulcerative, secretive and painful. Terbinafine was introduced without any improvement. Trichophyton mentagrophytes was isolated from the skin lesions. A novel homozygous mutation in CARD9 (R101L) was identified in the patient, resulting in impaired neutrophil fungal killing. Both parents, one brother (with persistent superficial but not deep dermatophytosis) and one sister were heterozygous for this mutation, while another brother was found to be homozygous for the CARD9 wild-type allele. This is the first report of CARD9 deficiency in Latin America.

Estudo multicêntrico, aberto, prospectivo de eficácia e tolerabilidade da desloratadina no tratamento da rinite alérgica / A multicentre, open-label, prospective study of the efficacy and tolerability of desloratadine in the treatment of allegic rhinitis

Introdução: A desioratadina, o principal metabólito da loratadína, é um novo anti- histamínico não sedante e tem se mostrado efetivo no controle de pacientes com rinite alérgica persistente (RAP) Objetivos: Avaliar a eficácia e a tolerabilidade da desioratadina (um comprimido de 5 mg), administrada em dose única diária por 14 dias, em pacientes com RAP (maiores de 12 anos de idade) de intensidade moderada@grave. Métodos: Este estudo, aberto e prospectivo, envolveu sete serviços universitários e avaliou 106 pacientes (12 a 55 anos, mediana 31,6 anos, 61,4por cento do sexo feminino) com diagnóstico médico de RAP Além de informações sobre a RAP (intensidade, duração, medicações utilizadas anteriormente, doenças associadas), foram pontuados os sintomas e sinais nasais (ETN): obstrução nasal, prurido nasal, espirros, rinorréia e os extranasais (ETEN): prurido ocular, hiperemia ocular, lacrimejamento e prurido em ouvídolpalato, conforme a intensidade (0-ausente a 3-íntenso) e com a soma dos dois escores se obteve o escore global (EGS). Além disso, os pacientes foram avaliados quanto à repercussão da RAP sobre a qualidade de vida. Os escores foram obtidos no início, 7 e 14 dias após o início do tratamento. A eficácia e a tolerabilidade da desloratadína pelo médico e pelo paciente, além do registro das reações adversas, também foram documentadas. Resultados: O esquema de tratamento foi respeitado por 98,5porcento dos pacientes. Hou- ve redução estatisticamente significante do ETN (8,31 x 3,85 x 3,40), do ETEN (4,85 x 1,54 x 1,4 1) e do EGS (13,16 x 5,39 x 4,8 1)já na primeira semana de tratamento e que se manteve na outra semana de avaliação. A qualidade de vida melhorou de modo signifi- cante durante o tratamento (9,91 x 6,07x 5,19). Ao final do tratamento, reações adversas foram relatadas por 4,7 porcento dos pacientes (sonolência e cefaléia) e de intensidade levei moderada na maioria deles. O tratamento foi considerado eficaz em 88porcento e 86 porcento dos pacientes, segundo médicos e pacientes, respectivamente. A tolerabilidade foi apontada como excelente e boa. Conclusões: A desloratadina (5 mgldia) foi eficaz no controle de sinais e sintomas de RAP e determinou melhora na qualidade de vida dos pacientes, foi bem tolerada e se mostrou segura.